RESUMO
Xanthogranulomatous pyelonephritis (XGP) is considered to be a rare variant of chronic pyelonephritis, which results in non-functioning kidneys in patients. The exact etiology of this disease is still unknown, and hence even its pathophysiology remains unclear. We present a case of a 27-year-old Saudi male patient who had been bed-bound with a known case of a congenital anomaly with severe kyphoscoliosis, bilateral lower limbs deformity with paraplegia, and a ventriculoperitoneal shunt since childhood. The patient was brought to the emergency department (ED) with right flank pain associated with fever and difficulty in breathing. The patient had a past medical history of recurrent urinary tract infection (UTI) with up to two incidences per year and renal stones. He had been recently discharged from the ICU of another hospital with sepsis due to UTI. An abdominal CT scan was performed, which showed a mass in the upper lobe of the right kidney measuring about 9 x 8 x 6 cm, suggestive of XGP. The final diagnosis was severe urosepsis secondary to right obstructive pyelonephritis. Patients with XGP usually present with nonspecific symptoms including back and abdominal pain, fever, UTI, and the condition is more common among middle-aged women. Ultimately, early detection and diagnosis, followed by prompt treatment with partial or total nephrectomy are associated witha good prognosis for patients with XGP.